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Chiari Malformation

What Is a Chiari Malformation?

A Chiari malformation is a birth defect in the back of the head where the brain and spinal cord connect. The condition is also called Arnold Chiari malformation.

  • Type I — the base of the skull and the upper spinal area are not formed properly. This type commonly goes unnoticed until problems arise in the adolescent or adult years of life.
  • Type II — part of the back of the brain shifts downward through the bottom of the skull area. This is the most common type of Chiari malformation and can be associated with spina bifida and hydrocephalus.
  • Type III — the back of the brain protrudes out of an opening in the back of the skull area.
  • Type IV — the back of the brain fails to develop normally.

Symptoms can vary, but the most common symptoms are:

  • Rapid, back and forth, eye movement
  • Developmental delays
  • Poor feeding and swallowing
  • Stiffness or pain in the neck or back of the head area
  • Weak cry
  • Breathing problems
  • Headaches
  • Decreased strength in the arms

Although the exact cause of Chiari malformation is unknown, it is thought that a problem during fetal development may cause the abnormal brain formation. Chiari malformation may be caused by exposure to harmful substances during fetal development or associated with genetic problems.

If a Chiari malformation occurs with other birth defects, the diagnosis may be made at birth. Other times, the diagnosis is made after the onset of specific signs and symptoms.

Your child's physician may ask about:

  • Prenatal and birth history
  • Family medical history
  • Developmental milestones

They will also do an examination which will include a measurement of the circumference of the child's head.

Diagnostic tests may be performed to confirm the diagnosis of a Chiari malformation, including:

UR Medicine's Treatments for Chiari Malformation

A Chiari Malformation is a life-long condition. UR experts offer specialized treatment that focuses on managing the condition. Specific treatment will vary, but may include:

  • Frequent physical examinations
  • Frequent diagnostic testing
  • Surgery to relieve increased pressure inside the head or neck area
  • Surgery to drain excess cerebral spinal fluid from the brain

Your provider may suggest genetic counseling to provide information on the recurrences for Chiari malformation and any available testing.

What Sets Us Apart?

UR Medicine is a world-class medical center and leading academic institution. Our providers offer outstanding patient-centered care and conduct breakthrough research in neurology.

Highly specialized newborn care is provided in our Neonatal Intensive Care Unit (NICU). Our NICU is a Level IV care facility and treats all neonatal medical and surgical problems, including advanced therapy for cardiac and pulmonary diseases, such as cardiac surgery and ECMO.

UR Medicine's Golisano Children's Hospital's Neonatology program has been recognized as one of the nation’s best in the U.S. News & World Report’s Best Children’s Hospital rankings.

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